pulmonary hypertension

was referred to a cardiologist in 2003 when a routine physical indicated a possible MI. Further testing revealed a soft systolic ejection murmur. The EKG was written off as a athletic heart after a stress test. (At the time, I was doing aerobics classes 8 hours a week and biking approx. 150 miles/week.) In 2004, I experienced swelling in my legs (+1to 2). An echo at that time showed mild regurgitation in both the mitral and aortic valve, moderate in the tricuspid, LVEF 55-65%, moderate pulmonary hypertension of 41mm, and mild leaflet thickening of the mitral valve. No follow up was recommended. A TEE at the time estimated the hypertension at 34mm. No follow up again. My annual in 2007 showed premature atrial contractions and a referral to my cardiologist followed. This time the edema in my ankles was noted again, another echo was done. LVEF>55%, again regurgitation at the aortic and mitral valves, plus now the pulmonic valve shows regurgitation. Tricuspid is moderate. Diastolic dysfunction noted and moderate pulmonary hypertension of 40-50mm. The right atrium is mildly dilated in size. The Holter monitor showed QRS 107530, with 57 Tachycardias, the longest lasting 36 minutes, 14 Brachycardias and 3316 PACs, 2 of which were couplets. I am also now diagnosed with ankylosing spondylitis and ulcerative colitis. I am experiencing some shortness of breath, swelling in my ankles, occasional nausea for no reason, and sometimes my hands and feet get extremely cold when exercising. I am currently on 10mg/kg remicade every 4 weeks. I have switched cardiologists because I didn't feel he was taking my concerns seriously. What is my prognosis? I've read that survival with pulmonary hypertension is in the range of 2-5 years. What procedures should I expect my new cardiologist to recommend? With the symptoms I currently have, what would you estimate my outcome to be? What medications, if any, are effective with my condition? How is pulmonary fibrosis diagnosed and treated? What about the diastolic dysfunction and its prognosis? What is causing the pulmonary hypertension?

Have you had any formal pulmonary function testing? It sounds like your heart may be the culprit in all of this, but your lung function is also really important to understand and get under control. I don't have any answers for you re your Q', your issues are complex. If I was in your shoes, I would be seeking a second or third opinion from a specialty heart and pulmonary hospital.

From a prognosis standpoint, remember its all just numbers, its not a definitive reality for you. You are an individual and the stats don't necessarily apply. I am recovering from a cardiomyopathy where I was told that I had a 33% chance of being on a heart transplant list, a 33% chance of serious residual heart probs and 33% chance of getting back to normal. I focussed on that last 33% and I basically set my mind to being in that third. For the most part it worked, I just have a leaky valve now and my heart muscle is looking great. I have other pulmonary issues - hence my suggestion that you get evaluated, liver issues (I was by numbers a quarter of the way to the liver transplant list) and some neuro issues too, despite the stats, I am here and doing just fine a year later.

How was the 40-50 mmhg range estimated? If it's done by echo there is up to a 10 mmhg margin of error. The only true way to get that value is by cath. Echo estimates are calculated by regurgitant volume from the tricuspid valve; then adding 10mmhg to that number. (assuming the RA is at 10mmhg) Does the echo show any stenosis of your pulmonary valve... infundibular or valve stenosis? If any pressure gradient exists it could cause both PA pressure and RA size increase.

An interesting article related to overestimation of PA pressure by echo alone.

http://www.erj.ersjournals.com/cgi/reprint/12/6/1476.pdf

Good luck with your research. I wish I could give you more help on the ankle swelling questions.

Pulmonary hypertension is only a diagnosis after all other function tests have been completed. Pulmonary hypertension diagnosis by echo is only an estimate based on current regurgitant flow from the tricuspid valve. I believe I read somewhere that the cutoff for high pulmonary pressure was raised to around 40 due to the high frequency of mild tricuspid insufficiency in the general population (normal people).

Tricuspid insufficiency by itself is not itself a disease and really never needs to be corrected; however further tests would need to show (if you indeed have moderate insuffiency) what the causes are.

What is the amount of insufficiency noted in your aortic/mitral and pulmonary valves? Keep in mind that trivial (trace) and mild regurgitation of those valves is a normal phenomenon captured on echos due to their high sensitivity. In fact more than 40% of the normal population has some amount of valvar insufficiency.

How much do you currently exercise? By your post it looks like you were quite active and could have atheletes heart (causing internal diameters of your chambers to increase) However the diastolic dysfunction confuses me.

What was your dr's response from the Holter results? Did he/she believe any of those could be due to anxiety/stress in that situation? I get all sorts of palpations when I'm stressed; when I finally relax myself I can finally feel normal again and go months without them.

Regarding the swelling in your ankles (sign of diastolic dysfunction) however could be due to the veins in your legs as well (Deep vein thrombosis) and not related to your heart. Your new cardiologist will probably be the best one to help you on that topic.

I read the article, but there are numerous other articles that state the echo estimates of PH are usually under estimated. The tricuspid regurgitation has gotten worse since three years ago, there was no dilation of the right atrium three years ago, and the PH estimate was less three years ago. So my conclusion is that something is going on here. I have trace regurgitation at the three other valves with no report of stenosis. Both estimates of PH were done by a doctor, not a technician, and at the same facility. Due to my symptoms, my current cardiologist is not doubting the diagnosis of PH.

Hi. I do know quite a bit about PH because I have it. It is true that the only true test is a right heart cath as echoes can be off by a huge amount, regardless of who reads them. That said, it is common to find PH with diastolic dysfunction....it is really pulmonary VENOUS hypertension not pulmonary ARTERIAL hypertension. The DD is causing the PH from the left heart, most likely. Very commonly DD is caused by high blood pressure, and the treatment for that is blood pressure meds. You will sometimes read that PH is very very rare, but it is only true idiopathic PAH that is really rare. Ph is also commonly associated with connective tissue disorders as you are aware. PAH drugs are getting better and better. I was on Tracleer myself for 15 months and had no side effects at all. Newer drugs are being developed all the time, but if yours is from the DD, which I would put money on, that is what needs to be treated and then the pressures will go down. Be careful of what you read on the internet about it as a lot of the info is outdated. You can get heart failure from the DD and it sounds like you may have a touch of that, which is treatable. The other valve regurg. may mean nothing. BTW, I am not a doctor but I am an RN! Try not to freak, it will all be ok, I promise! If you have any other questions, I will be glad to try and help.

My PFT test and CT scan of lungs both came back essentially normal. The enlargement of the heart has occurred within a period of the last 3 years, because I had an echocardio done in 2004. The tricuspid regurgitation is worse, and the pulmonary hypertension has gone from the 30-40 range to 40-50 in three years. I am definitely concerned about what to expect in my future. Yes, I am athletic, but the shortness of breath, ankle swelling, and excessive sweating are definitely worse than they have ever been in the past. My ankles are swollen in the morning when I awaken, so I don't think it is due to a vein insufficiency problem. Otherwise, they would be better in the morning and worsen upon standing throughout the day. I think it might be a related problem to the ankylosing spondylitis (which falls into the connective tissue disease category) that might be responsible for the lung pressures the doctors are seeing. I've read about the medications available for PH and they aren't really encouraging when it comes to side effects, etc. I'm really scared by all this.

Yes, your BP may go lower, but even if it is in the low 90's, as long as you can tolerate it, your docs may think it's ok. My BP also has always been quite low, and I am on an ACE for my CM. I can tolerate it as long as I stay above about 86 systolic! I just have to get up slowly from a sitting position so as to not feel lightheaded. DD, I understand, is being picked up more often now due to improved echo techniques/equipment. Cardios are studying it more in research studies as well.

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